7.

4 Adnexal tumors
Introduction:
Adnexal tumors are divided into tumors arising from the follicular epithelium, from sweat and sebaceous
glands. These tumours are usually benign. Their clinical appearance is often not diagnostic.
7.4.1 Tumors of the hair follicle
Introduction:
Signs of follicular differentiation: hair matrix, hair papillae, hair germs
7.4.1.1 Hair follicle nevus
Clinical signs:
• location: head, neck
• area of hypertrichosis
Histology:
Group of normal vellus hairs.
7.4.1.2 Aberrant follicle
Sometimes hair follicle may appear on a skin, where normaly no hair follicles are present (palm).
7.4.1.3 Trichofolliculoma
Histology:
Cystic tumor, lined by squamous epithelium, usually communicating with the surface. Within the wall of the
cyst there are small, malformed, well differentiated follicles with hair papilla. The tumor is surrounded by
fibrous tissue rich in fibroblasts.
Variant of this tumor with many sebaceous glands present as well is called sebaceous trichofolliculoma.
7.4.1.4 Trichoadenoma
Clinical signs:
• rare solitary tumor
• location: face
• size: up to 15 mm
Histology:
Groups of cysts, filled with keratin; granular layer is usually absent; epithelial strands connect the cysts; nests
of keratinocytes.
7.4.1.5 Dilated pore of Winer
Clinical signs:
• comedo-like benign tumor, rare
• location: face
Histology:
Dilated open follicular cystic cavity filled with keratin with irregularly proliferating rete ridges. The lesion is
sometimes pigmented.
7.4.1.6 Pilar sheath acanthoma
Clinical signs:
• papule, usually on the upper lip
• age: adults
Histology:
Dilated follicular infundibulum filled with keratin, acanthotic proliferation of the follicular epithelium; cells
are regular, lesion is benign.
7.4.1.7 Trichoepithelioma
Clinical signs:
• usually solitary papules, location: face
• multiple papules of the face in hereditary form, around the eyes, nasolabial folds
• tiny pearly papules
• color: skin color or yellowish
Histology:
The tumor consists of basophilic epithelium with peripheral palisading. Mitotic activity is usually minimal.
The slits between tumorous nests and the stroma, but rather between the stroma and uninvolved dermis.
Focal differentiation into hair bulbs with stromal reaction (and differentiation into the papilla). The tumor
doesn's usually reach depper than into 2/3 of the dermis (this differs from trichoblastoma).

7.4.1.8 Trichoblastoma
Histology:
Large tumor (reaching at least into half of the dermis) with variable structure (many types were described).
The tumor is characterized by proliferation of basaloid cells with focal differentiation into hair follicle,
especially by formation of hair papilla (epithelial and mesenchymal parts). Mitotic activity is present, but the
tumor is well demarcated, stroma is in contact with the epithelium (as opposed to bazalioma).
7.4.1.9 Desmoplastic trichoepithelioma
Clinical signs:
• small, centrally umbilicated tumor
• location: face
Histology:
Small nests and branching cords of squamous epithelium, small keratinous cysts, foci of keratinization, small
calcifications and ossifications. Stroma is collagenous. No retraction arteracts are present.
EMA is positive (positive in microadnexal carcinoma, negative in basal cell carcinoma), stromelysin 3 is
negative (positive in BCC and microadnexal carcinoma).
7.4.1.10 Tricholemmoma
Clinical signs:
• solitary tricholemmoma is a small papule, several mm in size
• location: usually the face
• multiple tricholemmomas are found in Cowden's disease
Histology:
Nests or larger solid tumors of pale, glycogen containing epithelium; peripheral palisading, usually thick
basement membrane.
7.4.1.10.1 Desmoplastic tricholemmoma
Histology:
Variant of the tricholemmoma, where multiple, irregular tumorous nests of pale epithelium with palisading
grow between dermal collagen.
7.4.1.11 Trichomatricom (pilomatrixom)
Clinical signs:
• firm, roundish, solitary subcutaneous nodule
• location: variable, especially the upper extremities or the face
• size: 5 – 30 mm
• age: any age, common in children
Histology:
Nodule consisting of dark, basophilic cells, often of high mitotic activity and pale, eosinophilic areas of
keratin and necrotic cells with eosinophilic cytoplasm and lost nucleus (ghost cells).
Dystrophic calcifications and inflammatory reaction are common, sometimes ossificatinons can be found.
7.4.1.11.1 Trichomatrical carcinoma
Introduction:
Rare malignant variant of pilomatricoma.
Pictures
Malignant pilomatricoma of sacral area. Infiltrative tumor after repeated local resections:
Trichomatricoma, malignant, HE 40x (5036) [zoomify]
Trichomatricoma, malignant, HE 40x (5037) [zoomify]
7.4.1.12 Folliculosebaceous hamartoma
Histology:
Intradermally located tumor, consisting of centrally located mesenchymal part (fat, blood vessels, fibrous
tissue), surrounded by malformed follicles with prominent sebaceous glands.
7.4.1.13 Tumor of the follicular infundibulum
Clinical signs:
Tumor located usually on the face, may be multiple (face, neck, trunk).
Histology:
Shallow acanthosis of pale, interconnected keratinocytes without atypia, PAS positive. The tumor is well
circumscribed.
7.4.2 Tumors of the sweat glands
Introduction:
Signs of sweat gland differentiation: tubules and ducts, secretion, sometimes papillary formations; eccrine or
apocrine secretion.
7.4.2.1 Eccrine nevus
Clinical signs:
• papular lesions or areas of hyperhidrosis
• very rare
Histology:
Groups of normal or enlarged eccrine sweat glands.
7.4.2.1.1 Eccrine angiomatous hamartoma
Histology:
Eccrine glands, blood vessels and sometimes nerve fibres.
7.4.2.2 Cylindroma
Clinical signs:
• location: usually scalp, face
• elastic, roundish tumors
• pink or skin colored papules
• often multiple

Histology:
Intraepidermal tumor consisting of (usually) multiple islands of tumorous cells, which are surrounded by
hyaline sheath and fit together like pieces of a jigsaw puzzle. The hyaline sheaths and hyaline droplets within
the tumor are PAS positive.
The tumor consists of two kinds of cells: smaller, on the periphery of the nests (with narrower cytoplasm),
and larger, more centrally located, with pale nucleus and eosinophilic cytoplasm.
The tumor can contain tiny tubules.
7.4.2.3 Eccrine spiradenoma
Clinical signs:
• intradermal nodule, size up to 2 cm, rarely more
• multiple, linearly arranged forms exist
• age: usualy young adults
• nodules are tender, sometimes painful
• localization: variable
Histology:
Intradermal tumorouus nodules, sharp circumscription, in scanning magnification may resemble lymphnodes.
Bands of tumorous cells with small, dark nuclei on the periphery and larger, pale nuclei in the centre. No
hyaline sheats around the bands are present. In addition tubules, PAS positive hyaline granules and scattered
lymphocytes are present.
Sometimes this tumor is mixed with cylindroma. In some tumors the cells form small rosettes.
7.4.2.4 Nodular hidradenoma
Histology:
Well circumscribed tumor, consisting of one or more tumorous nodes, solid, cystic, tubular.
Solid areas contain several kinds of cells: pale (or sometimes clear) cells and dark (with eosinophilic
cytoplasm), sometimes squamoid and mucinous cells are present as well. Cystic areas usually contain
eosinophilic amorphous material. The lining is flat. Tubules are lined by cubic or cylindric epithelium.
Mitotic activity is present even in benign tumors, the diagnostic criteria of malignity are cellular
polymorphism and invasive growth.
Clear cell hidradenoma consists of pale or clear cells.
7.4.2.4.1 Cystic nodular hidradenoma
Histology:
Variant of nodular hidradenoma with prevalence of cysts (solid structures are present as well, as opposed to
apocrine hidrocystoma).
7.4.2.4.2 Apocrine hidradenocarcinoma
Clinical signs:
• large nodule
• location: almost anywhere
• age: over 50
• rare
Histology:
Large tumor with focal apocrine differentiation, infiltrative growth, mitotic activity, focal necroses.
Malignant conterpart of solid hidradenoma.
7.4.2.5 Syringoma
Clinical signs:
• location: periorbital skin, neck, genital; more common in women
• tiny, smooth nodules
• color: skin color or pink
• variants: linear, eruptive
• clinically may resemble eczematous dermatitis (specimens of syringoma come under
this diagnosis to pathology sometimes)
Histology:
The tumor consists of many tiny cellular nests and ducts, containing PAS positive material. The lining is flat
or two-layered. Focal keratinisation is often present. Small cellular nests are assymmetric, of tadpole shape.
The tumor is usually small, limited to the upper dermis.
7.4.2.6 Papillary eccrine adenoma
Clinical signs:
• well circumscribed intradermal tumor
• papule, size about 1 cm
• location: extremities
Histology:
Intradermal tumor, consisting of small cysts (some cysts are of middle size), filled by eosinophilic debris.
Two layered epithelial lining (at least focally), small papillary projections. Cuboidal cells, no squamos
differentiation (no granular layer, inner surface of the cysts is uneven). No mitotic activity.
7.4.2.7 Apocrine nevus
Clinical signs:
• location: thorax, especially axilla
Histology:
Groups of normal or enlarged sweat glands.
7.4.2.8 Mammary gland anal polyp
• anal polyp
Histology:
Fibrous stroma and ectopic breast tissue; positive reaction to estrogen receptors.
7.4.2.9 Chondroid syringoma
Clinical signs:
• firm subcutaneous nodules
• size up to 2 cm
Histology:
Roundish tumor, benign, often separated from surrounding dermis by a cleft. Also called mixed tumor of the
skin (usually contains areas of chondroid appearance). Two forms are recognized: tubular and branching
(apocrine) and form with small tubular lumina (probably eccrine).
Apocrine variant is more common; epithelium is high, basophilic, two layered epithelium; forming duct-like
and cystic structures and epithelial nests. Stroma contains mucin and often is of chondroid appearance.
Epithelium sometimes shows decapitation secretion.
Eccrine variant consists of roundish tubules lined with single layer of epithelial cells. Stroma contains
mucin; chondroid changes are less apparent than in apocrine type.
7.4.2.10 Syringocystadenoma papilliferum
Clinical signs:
• location: face, sometimes elsewhere
• papule or sometimes several, linearly arranged papules
• inborn malformation
• enlarges during puberty
Histology:
Invagination, lined in the depth with apocrine epithelium of two layers of cells with apocrine secretion. Near
to the surface the lining changes into squamous epithelium. Many papillary projections extend into the
lumen.
The dermis contains a dense inflammatory infilrate with many plasma cells.
Sometimes malformed follicles and sebaceous glands are found near the lesion. Vulgar verruca is sometimes
present as well.
7.4.2.11 Hidradenoma papilliferum
Clinical signs:
• location: perigenital and perianal region, in women only (esp. on labia maiora)
• size: several milimeters
Histology:
Well circumscribed, sometimes encapsulated cystic apocrine adenoma, located within the dermis. The tumor
contains tubules, cysts and especially many papillary projections, covered by one row of columnar cells.
7.4.2.12 Tubular apocrine adenoma
Clinical signs:
• location: variable
• size: 1 – 2 cm or more
Histology:
Apocrine glands, cysts and tubules; sometimes focal papillary structures; sometimes communication with the
infundibula.
7.4.2.13 Poroma
Introduction:
Eccrine poroma is a tumor of sweat glands, consisting mostly of basaloid cells growing in solid pattern.
Focally small ducts can be found. Three variants of this tumor are recognized: eccrine poroma with tumorous
nests broadly connected to the epidermis, hidroacanthoma simplex (with clonal grow limited to the
epidermis) and dermal duct tumor (dermal form with no connection to the epidermis). Malignant poromas
(porocarcinomas) are rare.
7.4.2.13.1 Eccrine poroma
Clinical signs:
 • location: soles (2/3 of all cases), hands, fingers and other locations
• age: usually adults
• papule, size up to 20 mm, usually solitary
Histology:
The tumor is located within the dermis, but communicates with the epidermis by broad bands of tumorous
epithelium. the cells are basaloid: roundish, regular, paler than normal keratinocytes. The borders between
the tumor and the epidermis is usually easy to recognize. Small ducts, small foci of keratinisation and areas
of regression with nuclear pyknosis are usually present.

7.4.2.13.2 Hidroacanthoma simplex

Histology:
Clonal variant of the eccrine poroma, where basaloid, pale (glycogen), round cells form intraepidermal nests.
The epidermis is usually acanthotic. Differential diagnosis of this tumor against clonal seborrhoic keratosis is
difficult (duct formation, location —  seborroic keratoses do not appear on soles).
7.4.2.13.3 Dermal duct tumor
Histology:
One or more round dermal nodules composed of basaloid, regular, pale cells. Focally tiny ducts with
eosinophilic cuticle and keratinisation (squamous eddies) are formed. The tumor may be partially cystic.
7.4.2.13.4 Poroma, malignant (porocarcinoma)
Clinical signs:
• location: extremities (legs, feet)
• age: adult
• nodule, plaque, ulcerated tumor
• multiple cutaneous metastases may develop
Histology:
Poroma cells, cellular polymorphism, mitotic activity, hyperchromatic nuclei, irregular tumorous nodules.
7.4.2.13.5 Syringofibroadenoma
Clinical signs:
• hyperkeratotic papule
• location: extremity
• size: up to several centimeters
• rare tumor
Histology:
Anastomosing epithelial strands of basaloid cells communicating with the epidermis and with occassional
formation of lumina. Eccrine and apocrine variants exist.
7.4.2.13.6 Intraepidermal epithelioma of Borst Jadassohn
Histology:
Nests of intraepidermal tumorous cells, originally described as intraepidermal basalioma, are now considered
to be clonal seborrhoic keratosis or intraepidermal poroma (hidroacanthoma simplex).
7.4.2.14 Microcystic adnexal carcinoma
Clinical signs:
• location: head (upper lip, chin, cheek)
• deep invasion, local aggresivity
• no metastases
Histology:
Large, deeply infiltrating tumor, consisting of epithelial nests, ducts, glandular structures. Sometimes focal
hair follicle differentiation. Mitoses are rare. Perineural spread is sometimes present.
7.4.2.15 Adenoid cystic carcinoma
Clinical signs:
• very rare in the skin
 • reccurences occur often, but metastases are rare
Histology:
Masses of small, regular, basophilic cells form cribriform structures and small cystic spaces filled with
mucin. These spaces are lined by cuboid or flattened epithelium. Infiltrative growth, low or none mitotic
activity, sometimes perineural spread.
Resembles adenoid basalioma, but shows no peripheral palisading on the periphery of the tumorous nests.
The cells of adenoid cystic carcinoma are positive for carcinoembryonal antigen and S100 (basalioma cells
are negative).
7.4.2.16 Eccrine carcinoma
Clinical signs:
• localization: face, sometimes elsewhere
• skin nodule, growing slowly at first, later infiltrative
• may ulcerate
• rare
Histology:
Infiltrative growing tumor, consisting of irregular nests of atypical cells. Focally the differentiation towards
eccrine ducts is present. Variable desmoplasia.
7.4.3 Sebaceous tumors
Introduction:
Benign and malignant processes of sebaceous epithelium (hyperplasia, benign and malignant tumors).
Processes, in which (cytologically) normal sebaceous glands form a part of complex tumor are mentioned
elsewhere (sebaceous nevus, folliculosebaceous hamartoma etc.)
7.4.3.1 Ectopic sebaceous glands
Clinical signs:
• location: lip, buccal mucosa — Fordyce's spot
• location: areola mammae — Montgomery's tubercle
Histology:
Normal sebaceous glands not connected to any hair follicle.
7.4.3.2 Nevus sebaceus (of Jadassohn)
Introduction:
Tissue malformation, mentioned in the chapter on organoid nevi and tissue malformations
7.4.3.3 Sebaceous hyperplasia
Clinical signs:
• location: face (forhead, face)
• persons of middle age
• one or several soft papules, size less than 3 mm, often with central dimple
Histology:
The lesion consists of hyperplastic, enlarged sebaceous gland and the duct leading to the surface. Sebaceous
epithelium is mature. If immature, basaloid cells are present, than they are located on the periphery of the
lobules, and their number is low.
7.4.3.3.1 Sebaceous hyperplasia of the penis
7.4.3.4 Sebaceous adenoma
Clinical signs:
• location: face, scalp
• papule or nodule of yellowish color
• adenoma sebaceum is something else, namely angiofibroma in tuberous sclerosis
Histology:
Resembles a group of enlarged sebaceous glands; immature basaloid cells are on the periphery.
7.4.3.5 Sebaceoma, sebaceous epithelioma
Clinical signs:
 • slowly growing papules
Histology:
Tumor with focal sebaceous differentiation; more or less mature sebaceous cells are mixed randomly with
basophilic, immature sebaceous epithelia. Scattered mitoses may be present.
7.4.3.6 Muir-Torre syndrome
Clinical signs:
Association of mulitple sebaceous tumors (sebaceous hyperplasias, sebaceous adenomas and carcinomas)
and/or keratoacanthomas with visceral carcinomas (often of the colon).
7.4.4 Combined adnexal tumor
Histology:
Benign, roundish tumor consisting of areas showing areas of eccrine (or apocrine) and trichoepithelial
differentiation.
7.4.5 Cowden syndrome
Clinical signs:
• inheritance: autosomal dominant, chromosomal aberration on 10q23
• variable expression
• multiple hamartomas
• danger of malignant transformation (skin, breast, thyroid)
• age: manifestation starts at second or third decade
Histology:
• Skin and mucosal lesions:
o trichilemmomas, inverted follicular keratoses, tumors of follicular
infundibulum of the face
o acral keratoses (dorsal aspect)
o palmoplantar keratosis
o papillomatous papules of the face (6 papules, out of it 3 trichilemmomas)
o mucous membranes: fibromas, lingua scrotalis
• Main criteria:
o carcinoma of the breast (ductal carcinoma)
o carcinoma of the thyroid gland (follicular carcinoma)
o makrocephaly
o Lhermite-Duclos disease (hamartomas of the cerebellum)
o endometrial carcinomas
• Minor criteria:
o thyroid gland disorders
o low IQ
o hamartomas of the GIT
o fibrocystic disease of the breast
o lipomas, fibromas
o genitourinary tumors (Grawitz)